Article Text
Scientific letters
The mechanism of formation of pulmonary arteriovenous malformations associated with the classic Glenn shunt (superior cavopulmonary anastomosis)
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- ALK-1, activin receptor-like kinase 1, ET-1, endothelin 1
- HHT, hereditary haemorrhagic telangiectasia
- NO, nitric oxide
- PAVMS, pulmonary arteriovenous malformations
- TGF-β, transforming growth factor β
Anastomosis of the superior vena cava to the right pulmonary artery (classic Glenn shunt), although initially a successful procedure, is complicated by the development of troublesome diffuse microvascular and potentially lethal macrovascular pulmonary arteriovenous malformations (PAVMS) in up to 25% of cases. The pathogenesis of PAVMS is unclear but has been tentatively attributed to either the absence of pulsatile pulmonary blood flow or the absence of hepatoenteric effluent in the right lung.1 Evidence favours the latter suggestion. Firstly, other forms of cavopulmonary connection result in loss …